A rare small cell carcinoma with neurosecretory granules arising in an ovarian dermoid cyst with 7 years survival after conservative surgery and adjuvant chemotherapy with cisplatin, adriamycin, and cyclophosphamide is described. Light microscopy showed the typical uniformly small cells with hyperchromic nuclei and scanty basophilic cytoplasm within the mature cystic teratoma. Although none of the immunohistochemical stains were reactive, electron microscopy demonstrated the membrane-bound neurosecretory granules in some tumor cells. The related literature is reviewed and the issues concerning treatment options in this unusual malignancy are discussed.